cicatricisial alopecia

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TUTORIAL PRESENTATION CICATRICIAL ALOPECIA

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LICHEN PLANOPILARIS CHRONIC CUTANEOUS LUPUS ERYTHEMATOUS CENTRAL CENTRIFUGAL CICATRICAL ALOPECIA PSEUDOPELADE OF BROCQ ALOPECIA MUCINOSIS KERATOSIS PILARIS SPINULOSA DECALVANS

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TUTORIAL PRESENTATION

CICATRICIAL ALOPECIA

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Definition:Permanent area of hair loss associated with destruction of hair follicles

Pilosebaceous structures are replaced by fibrous tracts Classified as:

1. Primary 2. Secondary

Trauma Sclerosing disorders Granulomatous disorders Infections Neoplastic

3. Developmental/Hereditary PCA more common than SCA (4:1)

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ANOTHER CLASSIFICATION PROPOSED TO FACILITATE DETERMINATION OF MOST SUITABLE SURGICAL CORRECTIVE THERAPIES FOR CA :

stable - Traumatic Aplasia cutis CCCA

unstable – Lymphocytic Neutrophilic Mixed Infections Congenital Neoplastic

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PATHOGENESIS HFSCs destruction theories Impairment of self maintenance of HFSCs Alteration of lipid metabolism Neurogenic inflammation theory Environment factors Genetic factors

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PPAR DEFICIENCY CAUSES LOSS OF PEROXISOME BIOGENESIS, DEREGULATES LIPID METABOLISM, AND PRODUCES PROINFLAMMATORY LIPIDS THAT TRIGGER INFLAMMATORY RESPONSE THAT IN TURN CAUSES TISSUE DAMAGE AND PERMANENT HAIR LOSS

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NORMALIN CICATRICIAL

ALOPECIA

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APPROACH TO THE PATIENT

History Onset Presence of- pruritus, irritation, pain, erythema and/or

drainage from the scalp Evaluate for autoimmune disease, systemic illness,

infections, neoplasms, associated inflammatory skin disease and radiation treatment or burns

Drug intake Clinical Findings

loss of follicular ostia erythema, scaling, pustules, scalp bogginess and

compound follicles (polytrichia) Biopsy

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Non-scarring hair

Scarring hair loss

Incidence More common Less common

Erythema/scaling/pustules

-/+ +

Atrophy absent present

Loss of follicular openings

absent present

Tufted hair absent present

Course regrowth is quite common

no regrowth

Prognosis generally favourable

generally unfavourable

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PRIMARY CICATRICAL ALOPECIA

Diagnosis in which lymphocytes predominates includes:

LICHEN PLANOPILARISCHRONIC CUTANEOUS LUPUS ERYTHEMATOUSCENTRAL CENTRIFUGAL CICATRICAL ALOPECIAPSEUDOPELADE OF BROCQALOPECIA MUCINOSISKERATOSIS PILARIS SPINULOSA DECALVANS

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GRAHAM-LITTLE PICCARDI LASSUEUR SYNDROMEAFFECTS WOMEN BETWEEN 30-70 YRSSYNDROME CHARACTERIZED BY:PROGRESSIVE CICATRICIAL ALOPECIA OF SCALP,NON SCARRING ALOPECIA OF AXILLA AND PUBIC AREA AND KERATOSIS PILARIS

Classic lichen planus40% of pt have skin manifestations

C/Fs: violaceous papules,erythema scaling

Papules replaced by follicular plugs

Plugs shed and finally atrophic,smooth,scarred area remains

Pt commonly presents with pseudopelade like patches

Frontal Fibrosing AlopeciaResembles AGA with frontal recession

C/Fs:perifollicular erythema and hyperkeratoses at marginal hairline

Slow progressive disease

Typically occurs in Post-menopausal women

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Central centrifugal cicatricial alopeciahot comb alopecia, follicular degeneration syndrome, pseudopelade in African Americans central elliptical pseudopelade

Premature disintegration of inner root sheath epithelium occurs

Begins as single focus over vertex of scalp and then spread centrifugally

Pseudopelade

Idiopathic,chronic,slowly progressive

Patchy cicatricial alopecia that occur without any evidence of inflammation

footprints in the snow

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Chronic Cutaneous Lupus Erythematosus

30% have skin manifestationErythema, scaling and pigmentary changes are more pronounced Follicular plugging and adherent scale may be present. The “carpet tack” sign may be elicited with retraction of scale revealing keratotic spikes that correspond to follicular openings on undersurface

Keratosis follicularis spinulosa decalvans

X-Linked recessive SSAT gene defectErythema, plugging of eyebrow follicles follicular hyperkeratosis & prominent cuticles Ocular signs include blepharitis, ectropion, corneal dystrophy and photophobiaFocal PPK may be present

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Diagnosis in which neutrophils predominates includes:

FOLLICULITIS DECALVANSDISSECTING CELLULITIS OF SCALP

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Folliculitis decalvans

-Recurrent crops of follicular pustules that result in permanent epilation

-Staph aureus may be grown from pustules

-Pustular folliculitis followed by rounded patches of alopecia develop surrounded by crusting and few follicular pustules.

-Successive crops of pustules appear and are followed by progressive destruction of affected follicles

-Tufted folliculitis variant of folliculitis decalvans where circumscribed areas of scalp inflammation heal with scarring characterized by tufts of up to 15 hairs emerging from single orifice

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Perifolliculitis of scalp, deep and superficial abscesses in dermis, sinus tract formation and extensive scarring

Aetiology:staphylococci, streptococci and Pseudomonas may be cultured from various lesions

C/Fs:Painful, firm, skin-coloured nodules develop near vertex

Confluent nodules form tubular ridges with an irregular cerebriform pattern

Progressive scarring and permanent alopecia occur

Chronic condition with frequent acute exacerbations.

Dissecting cellulitis of the scalp

Perifolliculitis capitis abscedens et suffodiens

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Diagnoses in which a mix of cell types predominate are as follows:

ACNE KELOIDALISACNE NECROTICAEROSIVE PUSTULAR DERMATOSES OF SCALP

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Acne keloidalis

It occurs in males after puberty between the ages of 14 -25 yrs

C/Fs:Pts present with pustules, alopecia and hypertrophic scarring on posterior neck

Friction from the collar is often incriminated

Process begins with penetration of cut hair into

the skin as in pseudofolliculitis

Acne necrotica

More frequent in men than in women

30- 50 yrs

C/Fs:red itchy acneiform papules arise spontaneously on the front and sides of scalp

papules are usually centered around pilosebaceous unit 

Often umbilicated and rapidly transformed by necrosis into an adherent haemorrhagic crust which separates after 3 or 4 weeks to leave a permanent varioliform scar

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Particularly affects the elderly Precipitating factors:local trauma

sundamage,surgery, cryosurgery skin grafting and radiation therapy

Initially, a small area of scalp becomes red, crusted and irritable

crusting and superficial pustulation overlie a moist, eroded surface

As condition extends areas of activity coexist with areas of scarring.

Squamous carcinoma has developed in scars

Erosive pustular dermatosis of the scalp

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SECONDARY CICATRICIAL ALOPECIA

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GRANULOMATOUS DISORDERS

C/F:The oval atrophic plaques on the shins but may be seen on other parts of body including scalp.

The patches are glazed, yellowish often with conspicuous telangiectasia

Scarring may be dense. Clinical features in scalp vary

from large plaques of cicatricial alopecia to multiple small areas of scarring

Cutaneous sarcoidosis may produce plaques or nodules on scalp

Necrobiosis lipoidica, granuloma annulare and sarcoidosis

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Circumscribed scleroderma and linear morphoea

rare in the scalp

’en coup de sabre’ morphoea – is more common

Cicatricial pemphigoidWomen > men

disease predominantly affects ocular and/or genital mucous membrane

skin is involved in 40–50%

scalp involved in 10% of cases

SCLEROSING DISORDERS

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TRAUMATIC

Common in Afro-Caribbean hair styles

Due to sustained pull on hair roots

Folliculitis , hair casts reduction in hair density

with vellus hairs and sometimes broken hairs

Hair loss begins in temporal regions and in front of and above the ears but may involve other parts of scalp

CHILD: Scalp electrodes or infusion or forceps delivery or uterine rings in neonate can result in trauma.

ADULT:Brain surgery, gynaecological surgery in Trendelenburg position

Traction alopecia Medical trauma

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TRICHOTILLOMANIA

Behavioural disorder characterized by compulsive hair pulling Trichoteiromania : Compulsive hair rubbing Trichotemnomania : Compulsive hair cutting Hair is plucked most frequently from one frontoparietal region Patch of hair loss bizarre or angular pattern in which hairs

are twisted and broken at various distances from clinically normal scalp

H/P: Numerous empty canals , clefts in hair matrix, intraepithelial and perifollicular haemorrhages and intrafollicular pigment casts

Some follicles are severely damaged Follicular epithelium is separated from connective tissue sheath Trichomalacia - Injured follicles may form only soft, twisted hair

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TRACTION ALOPECIA. TRACTION FOLLICULITISIS COMMONLY ASSOCIATED

Syphilitic alopecia.

The scalp has moth-eaten appearance, eyebrow hair is absent there is rash on the cheek

Trichotillomania. Hairs are thin

and of different lengths

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TRACTION ALOPECIADERMATOPHYTE:INFECTION

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DIAGNOSIS

Dermoscopy/Trichoscopy - first-line, noninvasive method

Absence of follicular ostia in 100% cases even if it is not evident clinically

FFA :loss of orifices, perifollicular scale and feeble perifollicular erythema

Folliculitis decalvans :existence of micropustules and/or hair tufting with >=6 hairs

DLE: follicular red dots  LPP: hair tufting, violaceous-blue interfollicular area,

corresponding to pigment incontinence Lipedematous alopecia: linear area of telangiectasia within

scalp creases, possibly caused by compression of the superficial blood capillaries

Scalp sarcoidosis: orange spots seen(round, well-formed granulomas in superficial dermis)

Traction alopecia: Hair casts

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Reflectance confocal microscopy Microscopic imaging of superficial layers of skin

down to superficial reticular dermis with resolution at cellular level close to conventional histopathology

May also help in choosing most appropriate biopsy site for more informative histology

HistopathologyDirect immunofluorescenceMicroarray analysis

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LUPUS ERYTHEMATOUS: H/P:VACUOLAR INTERFACE ALTERATION OF FOLLICULAR EPITHELIUM, SCATTERING OF DYSKERATOTIC KERATINOCYTES, VARIABLY DENSE PERIADNEXAL, PERIFOLLICULAR (UPPER PORTION), PERIVASCULARAND INTERSTITIAL LYMPHOCYTIC INFILTRATE WITH DERMAL MUCIN, ATROPHY OF SEBACEOUS GLANDS AND FOLLICULAR PLUGGING. EPIDERMIS MAY BE ATROPHIED WITH VACUOLAR INTERFACE CHANGES. CONCENTRIC LAMELLAR FIBROSIS AROUND THE FOLLICLE IN END STAGES

follicular plugging, superficial,deep perivascular and periappendageal lymphocytic infiltrate

There is linear staining of deposits of complement (C3), IgM and IgG on the basement membrane in more than 80% of cases of LE

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LICHEN PLANUS: ACTIVE : FOLLICULAR LYMPHOCYTIC INTERFACE DERMATITIS WITH DENSE BAND LIKE LYMPHOCYTES AROUND UPPER FOLLICLE & INFUNDIBULUM OBSCURING DEJ, INFUNDIBULAR HYPERKERATOSIS AND HYPERGRANULOSIS, CYTOID BODIES SCATTERED ALONG THE BMZ, ABSENT OR ATROPHIC SEBACEOUS GLANDS WITH OR WITHOUT PIGMENTARY INCONTINENCE. END –STAGE : LONGITUDINAL TRACTS OF FIBROSIS,LAMELLAR FIBROSIS,EPIDERMAL ATROPHYDIF:‘SHAGGY’ OR ‘PATCHY’ DEPOSITION OF FIBRINOGEN AND CLUMPED IGM OR LESS COMMONLY IGA AND C3 DEPOSITS ARE SEEN ALONG FOLLICULAR BMZ

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Variably dense perifollicular lymphocytic infiltrate in early stage, followed by eccentric atrophy of follicle infundibular epithelium, concentric lamellar fibrosis around upper follicle and loss of sebaceous gland in later stage

Elastin stains reveal dense elastic tissue cuffing a broad fibrotic follicular tract in advanced disease

thinned out epidermis with total loss of hair follicles, replaced by collagen

Pseudopelade of Brocq

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Central centrifugal cicatricial alopecia :Earliest feature is premature disintegration of IRS resulting in outward migration of hair shaft through ORS at level of isthmus. Lamellar fibroplasias and lymphocytic inflammation surround the follicle at this level, resulting in follicular destruction and fibrous tract formation.

Alopecia mucinosa :Mucinous degeneration of ORS and sebaceous glands. A perifollicular lymphocytic infiltrate often with eosinophils and histocytes

Keratosis follicularis spinulosa decalvans :Compact hyperkeratosis, hypergranulosis of upper follicular epithelium with superficial intrafollicular and peri-follicular edema in early stage whereas in advanced stage there is concentric perifollicular, horizontal adventitial lamellar fibrosis and scarred follicular tracts

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Folliculitis decalvans: Acneiform dilatation with perifollicular neutrophilic inflammation- later mixed inflammatory infiltrate of neutrophils, lymphocytes, plasma cells. Follicular rupture,foreign-body giant cell granuloma formation around exposed hair shaft fragments. In burnt outstage, follicular and adventitial fibrosis is seen

Dissecting cellulitis of scalp: Infundibular acneiform distention with intrafollicular and perifollicular neutrophilic infiltration, abscess formation,sinus tracts

Acne keloidalis : perifollicular and intrafollicular lymphoplasmacytic infiltrate at the level of sebaceous glands -complete follicular destruction occurs with loss of sebaceous glands and dermal fibrosis

Acne necrotica:dense perivascular and perifollicular lymphocytic infiltrate with prominent sub-epidermal edema. Necrosis of individual keratinocyte is seen initially and is followed by confluent necrosis of the central follicle and interfollicular epidermis

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TREATMENT

Aim of treatment currently focuses reduction of symptoms and to reduce or stop progression of disease.

LMPCA with immunosuppression NMPCA with antimicrobials or dapsone

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First line Class I or II potent topical

corticosteroids I/L steroid inj (10 mg/ml max 2 ml,

every 4-6 weeks) Results are assessed till 8 weeks, if

no response shift to next levelSecond line Antimalarials (HCQ 200-400 mg/day,

clinical effect in 4-8 weeks, continued till 3-6 mths)

Oral corticosteroids (1 mg/kg, for initial actively progressing disease, tapered over 8 weeks)

Retinoids (acitretin and Isotretinoin 10-40 mg/day)

Third line Thalidomide, topical

immunomodulators , oral vit E, gold, dapsone,MMF,methotrexate, azathioprine,clofazamine,systemic or intralesional INFα2, monoclonal anti-CD4 antibodies, topical 5-FU, topical tazarotene imiquimod.

First line Potent topical corticosteriods I/L Triamcinolone acetonide

Second line Oral corticosteroids Oral cyclosporine(4-5 mg/kg for 4-

6 mths) Topical cyclosporine (oily solution,

applied twice daily for initial 3 mths and once daily for further 3 mths)

Oral tetracycline

Third line Retinoids ,Antimalarials,MMF(500

mg twice daily) Others:

Thalidomide,griseofulvin,low molecular weight heparin (s/c injections 3 mg once weekly), exicmer laser

Newer therapies PPARγ agonist like

thiozolidinediones

DLE LPP

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Intralesional triamcinolone acetonide

Finasteride (2.5 mg OD) Oral corticosteroids Antimalarials Topical corticosteroids with topical

minoxidil Oral retinoids

GLP Topical and intralesional

corticosteroids Oral ciclosporin Systemic corticosteroids Topical tacrolimus

PPB Potent topical corticosteroids(±)

First line Potent topical corticosteriodsSecond line I/L triamcinolone Minocycline DapsoneThird line Systemic steroids,isotretinoin,

antimalarials,PUVA,interferon α-2b+Interferon γ,superficial X-rays

KFSD Oral antibiotics Dapsone Oral retinoids Laser epilation

FFA Alopecia mucinosa

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First line Oral±topical antibioticsSecond line Oral rifampicin (300 mg BD)

+oral clindamycin (300 mg BD) Rifampicin+ (doxycycline/ciprofl

oxacin/clarithromycin) Oral rifampicin+topical

antibiotics

Third line Oral fusidic acid Oral zinc Dapsone Oral cyclosporine Excision,laser,radiotherapy,i/m

Human immunoglobulin

First line Oral isotretinoin Oral isotretinoin+i/l

triamcinolone acetonideSecond line Oral antibiotics+topical

antibiotics/topical retinoids Aspiration and i/l triamcinolone

acetonideThird line Low dose corticosteroids Colchicine Dapsone Excision and skin grafting Lasers and radiotherapy

FD Dissecting cellulitis of scalp

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First line Potent topical steroid Oral antibiotics+topical

steroids/intralesional triamcinolone

Second line Surgical excision CO2 laser Diode laser hair epilationThird line Radiotherapy Isotretinoin

Oral antibiotics Oral isotretinoin I/L triamcinolone

Erosive pustular dermatosis of scalp

Topical corticosteroids Topical

immunomodulators Calcipotriol cream Oral Isotretinoin

Acne keloidalis nuchae Acne necrotica varioliformis

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Surgical treatment of scarring alopecia includes : hair transplantation scalp reduction or alopecia reduction surgeries tissue expansion flap surgeries

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CONCLUSION

CA is ‘trichology emergency’ situation, in which lack of prompt and early treatment will lead to the inevitable loss of hair follicles along with permanent scarring.

Newer pathogenesis has given the platform for development of emerging treatment modalities.

But still great deal of research is required in this field, may be developing viable stem cell therapies or bioengineered human hair follicles are the answers to it in future

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THANK YOU